Superior vena cava syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Superior vena cava syndrome (SVCS) is an array of symptoms caused by the impairment of blood flow through the superior vena cava (SVC) to the right atrium. Symptoms that prompt suspicion of this syndrome include dyspnea, coughing, and swelling of the face, neck, upper trunk, and extremities. In rare instances, patients may complain of hoarseness, chest pain, dysphagia, and hemoptysis. Physical signs that may be noted on presentation are neck vein distention, thoracic vein distention, edema of the face or upper extremities, plethora, and tachypnea. Rarely, cyanosis, Horner syndrome, and a paralyzed vocal cord may also be present.

Historical Perspective

The Scottish obstetrician and anatomist, William Hunter, first described the superior vena cava syndrome in 1757, noting it as a complication of a syphilitic aortic aneurysm. In the past, it was a medical emergency and empiric radiation was given to shrink the tumor. With the advent of better medical therapy for some lung cancers and lymphoma and the low morbidity associated with diagnostic procedures, this approach has fallen out of favor.

Pathophysiology

The superior vena cava (SVC) is the major blood vessel for drainage of venous blood from the head, neck, upper extremities, and upper thorax to the heart. Obstruction of the superior vena cava (SVC) may be caused by neoplastic invasion of the venous wall associated with intravascular thrombosis, enlarged nodes, enlarged ascending aorta, or more simply, by extrinsic pressure of a tumor mass against the thin-walled superior vena cava (SVC) which leads to the development of SVC syndrome.

Causes

Superior vena cava syndrome may be caused by obstruction of the superior vena cava (SVC) by neoplastic invasion of the venous wall associated with intravascular thrombosis, enlarged nodes, enlarged ascending aorta, or more simply, by extrinsic pressure of a tumor mass against the thin-walled superior vena cava (SVC).

Differentiating Superior Vena Cava Syndrome from other Diseases

Superior vena cava syndrome should be differentiated from other causes of dyspnea and jugular venous distention, such as, cardiac tamponade, chronic obstructive pulmonary disease, mediastinitis, pneumonia, acute respiratory distress syndrome, syphilis, and tuberculosis.

Epidemiology and Demographics

Most SVC syndromes in the present day are related to malignancy. An underlying malignancy is found in approximately 90% of patients.